Systemic lupus erythematosus

(SLE)
 Definition

SLE is an inflammatory autoimmune

disorder that may affect multiple organ
systems.
 Introduction
Prevalence of SLE in China is 1/1000
Common in women of child-bearing years
Chronic systemic autoimmune disease in which
multiple organs undergo damage
Various autoantibodies exist in SLE patient’s
sera
SLE is of characteristic of remission and flare-
up alternately
 Systemic lupus erythematosus

Damage
Many in multiple
autoantibodies organ
systems
 Etiology

1. Genetic factors : SLE is known to occur in

families.
2. Environmental factors: may induce the onset
and flare.
ultraviolet light 、 stress 、
drug 、 pathogens
3. Hormonal factors: estrogen
 Pathogenesis

1. Poly-clonal B cell activation giving rise to the

production of a plethora of autoantibodies
2. Antibodies to other cell structures like
erythrocytes, platelets and phospholipids
3. Immune complex deposit in joints, small
vessel or glomeruli
4. Activated complement system cause intense
inflammation and tissue injury
 Pathology

Inflammation and vascular abnormalities

IC deposition

Inflammation Ischemia of
and local tissue
necrosis And dysfunction
Causing thrombosis
Abs invasion directly
Hematoxylin bodies
Onion skin lesions

SLE 的特征性病理改变：洋葱皮样改变 ( 脾中央动脉 )

WHO has classified lupus nephritis into
6 grades ：

I Normal glomeruli
II Pure mesangial nephritis
III Focal segmental glomerulonephritis
IV Diffuse proliferative glomerulonephritis
V Membranous glomerulonephritis
VI Advanced sclerosing glomerulonephritis
Transformation of histological type does occur.
 Clinical manifestations

1.General symptoms ： fever, fatigue,

weight loss
2.Cutaneous manifestations ： 80 ％ of Butterfly rash

people have rash

Butterfly rash
Discoid lupus erythematosus
Palm or peri-ungual erythema

Erythema on palm
Malar rash
Discoid rash
photosensitivity
livedo reticularis
oral ulcers 　
livedo reticularis
alopecia
Raynaud‘s phenomenon 　
　　

Raynaud's phenomenon
3. Serositis ： pleura effusion, pericardial effusion
4. Musculoskeletal manifestations
arthritis ， ischemic necrosis of bone, myositis
5. lupus nephritis, LN 　
renal involvement is present in approximately
75 ％ patients: proteinuria, hematuria and
casts,
chronic nephritis, nephrotic syndrome.
6. Cardiovasular manifestations pericarditis,
myocarditis, 　 endocarditis ， arrhythmia,
cardiac failure
7. Pulmontary manifestations
Pleuritis with or without pleural effusion in
approximately 35% patients
Pneumonitis
Interstitial lung disease
Pulmonary hypertension
8. Nervous system manifestations

headache

psychosis Vomiting

Cognitive
dysfunction hemiplegia

epilepsy
9. Gastrointestinal manifestations

Loss of appetite, abdominal pain,

vomiting, diarrhea, ascite
Liver abnormalities
Acute abdomen: pancreatitis,
intestinal ischemia and
obstruction which are the results of
vasculitis involving the intestine
10. Hematologic manifestations

Anemia
Leukopenia, lymphopenia
Thrombocytopenia
Painless lymphadenopathy
splenomegaly
11. Ocular manifestations
Nonspecific conjunctivitis, 　 retinal
vasculitis and optic neuritis
 Laboratory findings

General auto- Lupus band Radiographic

complement Renal biopsy
examination antibodies test Examination

Anti-
phospholipid
C3 Represent diagnosis MRI
Blood Rt ANAs
Urine Rt C4 disease therapy CT
Abs against CH50 activity
ESR prognosis
tissue
or cell

ANA
RNP
Sm SSA
ANAs dsDNA
rRNP SSB
ENAs
 Diagnosis
The 1997 revised criteria for the classification of SLE
1.Malar rash
2.Discoid rash
A patient is classified as
3.Photosensitivity
having SLE if any 4 or more of
4.Oral ulcers 11 criteria are met, with 85 ％
5.Arthritis specificity and 95% sensitivity.
6.Serositis
7.Renal disorder
8.Neurologic disorder
9.Hematologic disorder
10.Immunologic disorder
11.Positive antinuclear antibodies
 Differential diagnosis
Rheumatoid arthritis
Various dermatitis
Epilepsy
Mental illness
ITP
Primary glomerulonephritis
Other connective diseases
Drug-induced SLE
 Treatment

General therapy:
against sun exposure
rest
Corticosteroids
Immunosuppressives
IVIG
Control complications
 Treatment
Corticosteroids
Prednisone ： 0.5- 1mg/kg/d ， for 8 weeks 　 and 　 then
decrease the dosage. Decreasing 10% every 1-2 weeks,
with 5-10mg for continuous therapy
Pulse intravenous corticosteroids ： Methylprednisolone
500-1000mg/d for 3 days--- Prednisone ,orally. This is
used for acute renal failure, lupus neuropsychosis, severe
hemolytic anemia.
Immunosuppressive agents
Pulse cyclophosphamide: 0.5-1.0g/m2 ,every
3-4 weeks for 6 times
CTX orally, 1-2mg/kg/d
Methotrexate (MTX) 10-30mg/w orally
Azathioprine(AZA) 1-2mg/kg/d orally
Cyclosporine(CsA) 5mg/kg/d orally
Mycophenolate mofetil(MMF) 1-2g/d orally
Hydroxychloroquine(HCQ) 0.4g/d orally
IVIG: 0.4/kg/d for 3-5 days.
Monoclonal antibodies: e.g.anti-CD20 Ab.
 Prognosis
• The prognosis for patients with SLE appears to
be considerably improved compared to the
past, the 10-year survival rates has exceeded
90%. It is closely related to the early diagnosis,
treatment and long term doctor-patient
interaction.
 Prognosis
• However, there are some in whom the disease
pursues a virulent course, leading to serious
impairment of vital organs such as lung ,heart,
brain, or kidney resulting in death.
 Prognosis
• Infections especially with opportunistic
organisms have become the leading cause of
death, followed by active SLE, chiefly due to
renal, cardiovascular disease.
• Accelerated atherosclerosis, attributed in part
to corticosteroid use, has been responsible for
a rise in late deaths due to myocardial
infarction.
 Summary
Basic pathology: vasculitis

a chronic systemic autoimmune

disease in which multiple organs
undergo damage with Various
autoantibodies

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